Oftalmoplejía completa secundaria a un infarto talámico paramediano bilateral / Complete ophthalmoplegia secondary to a bilateral paramedian thalamic infarct

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[Recurrent painful ophthalmoplegia secondary to polyostotic fibrous dysplasia of the maxillary sinuses with involvement of the superior orbital fissure]. / Oftalmoplejia dolorosa recurrente secundaria a displasia fibrosa poliostotica del seno maxilar con afectacion de la hendidura esfenoidal.

INTRODUCTION: Fibrous dysplasia is a bone disease that is usually accompanied by asymptomatic lesions but which may sometimes display neurological manifestations due to the involvement of the craniofacial bones. CASE REPORT: A 25-year-old female, with a history of migraines, who visited at the age of 18 due to pain in the right retro-ocular and maxillary region, although with characteristics that were unlike those of her usual migraines, and which was associated with ipsilateral ophthalmoparesis. The condition had a self-limiting course and responded well to corticoids, although it was recurring. Examination revealed intense pain on palpation of the right-hand maxillary sinus and incomplete paralysis of the ipsilateral oculomotor nerve with palpebral ptosis. Results of complementary studies were normal, except for magnetic resonance imaging of the head and computerised axial tomography of the face, which revealed an expansive lesion with involvement of the right superior maxillary sinus and the greater wing of the sphenoid bone, with probable compromise of the superior orbital fissure, consistent with the diagnosis of fibrous dysplasia, which was confirmed by means of a pathology study. CONCLUSIONS: Fibrous dysplasia is a benign bone disorder, of unknown causation, in which normal bone tissue is replaced by amorphous conjunctive tissue. There is sometimes craniofacial involvement and a hypertrophic bone mass is formed which can fill the paranasal sinuses and the orbit, resulting in exophthalmus and visual disorders. To date the scientific literature does not include any reports of this disease manifesting as bouts of recurrent painful ophthalmoparesis which responds to corticoids, as happened in the case of our patient.

[Diagnostic value of cardiac 123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy in Lewy body disorders]. / Valor diagnóstico de la gammagrafía cardíaca con 123I-metaiodobenzilguanidina en las enfermedades con cuerpos de Lewy.

INTRODUCTION: Lewy body disorders such as Parkinson's disease (PD) and Lewy body dementia (LBD) are associated with cardiac sympathetic denervation, which can be visualized on 123I-MIBG scintigraphy. Our objectives were to study the diagnostic value of this technique in Lewy body disorders and its relationship with PD clinical variables. PATIENTS AND METHODS: We studied 90 patients: 51 with PD, 19 with LBD, 9 with multiple system atrophy (MSA) and 11 controls. Scintigraphy images were qualitatively evaluated and early and delayed heart-to-mediastinum ratios (HMR) were calculated. The main confounding factors (ischemic heart disease, diabetes, hypertension and drugs) were controlled by multivariate linear regression analysis. We investigated correlations between scintigraphy variables and PD variables. RESULTS: The delayed HMR, which showed better discriminative ability was 2.03 +/- 0.32 in controls, 1.37 +/- 0.30 in PD (p<0.001 vs controls), 1.47+/-0.45 in LBD (p=0.001 vs controls) and 1.69+/-0.28 in MSA (p=0.02 vs controls; p=0.004 vs PD). This ratio was influenced by PD/LBD diagnosis (beta= -0.638; p<0.001) and to a lesser degree, by ischemic heart disease (beta= -0.244; p=0.028). Optimal cut-off value between PD/LBD and controls was 1.71 (83% sensitivity and 82% specificity). Within the PD group, those with a family history of PD/LB showed higher delayed HMR values (1.65+/-0.34 vs 1.30+/-0.24 without history; p<0.001) and proportion with normal scintigraphy (56% vs 5%; p=0.001). CONCLUSIONS: Cardiac 123I-MIBG scintigraphy is useful in the diagnosis of Lewy body disorders, although its value in PD is conditioned by having a family history of PD.

Valor diagnóstico de la gammagrafíacardíaca con 123I-metaiodobenzilguanidinaen las enfermedades con cuerpos de Lewy / Diagnostic value of cardiac 123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy in Lewy body disorders

Introducción. Enfermedades con cuerpos de Lewy (ECL), como laenfermedad de Parkinson (EP) y la demencia con cuerpos de Lewy(DCL), asocian una denervación simpática cardíaca que puede evidenciarsemediante gammagrafía con 123I-metaiodobenzilguanidina(123I-MIBG). Nuestros objetivos fueron estudiar su valor diagnósticoen las ECL y su relación con variables clínicas de la EP.Pacientes y métodos. Estudiamos 90 pacientes: 51 con EP, 19 conDCL, 9 con atrofia multisistémica (AMS) y 11 controles. Se realizó valoracióncualitativa de la gammagrafía y se calcularon los índices corazón/mediastino (ICM) precoz y tardío. Los principales factores deconfusión (cardiopatía isquémica, diabetes, hipertensión y fármacos)se controlaron mediante regresión lineal multivariante. Efectuamoscorrelaciones entre las variables gammagráficas y del grupo con EP.Resultados. El ICM tardío, con mayor capacidad discriminativa,fue 2,03±0,32 en los controles, 1,37±0,30 en EP (p<0,001 vs controles),1,47±0,45 en DCL (p=0,001 vs controles) y 1,69±0,28 enAMS (p=0,02 vs controles; p=0,004 vs EP). En este índice influía eldiagnóstico de ECL (ß=–0,638; p<0,001) y en menor grado la cardiopatíaisquémica (ß=–0,244; p=0,028). Identificamos el valor 1,71como mejor punto de corte entre ECL y controles (sensibilidad 83%y especificidad 82%). Dentro del grupo con EP, aquellos con antecedentesfamiliares de EP mostraron mayores ICM tardío (1,65±0,34 vs1,30±0,24 sin antecedentes; p<0,001) y proporción de gammagrafíasnormales (56% vs 5%; p=0,001).Conclusiones. La gammagrafía cardíaca con 123I-MIBG es útilen el diagnóstico de ECL, si bien, en la EP su valor está condicionadopor el hecho de tener historia familiar de la enfermedad (AU)

Introduction. Lewy body disorders such as Parkinson’s disease(PD) and Lewy body dementia (LBD) are associated withcardiac sympathetic denervation, which can be visualized on123I-MIBG scintigraphy. Our objectives were to study the diagnosticvalue of this technique in Lewy body disorders and its relationshipwith PD clinical variables.Patients and methods. We studied 90 patients: 51 with PD,19 with LBD, 9 with multiple system atrophy (MSA) and 11 controls.Scintigraphy images were qualitatively evaluated and earlyand delayed heart-to-mediastinum ratios (HMR) were calculated.The main confounding factors (ischemic heart disease, diabetes,hypertension and drugs) were controlled by multivariate linearregression analysis. We investigated correlations between scintigraphyvariables and PD variables.Results. The delayed HMR, which showed better discriminativeability was 2.03 ± 0.32 in controls, 1.37 ± 0.30 in PD(p<0.001 vs controls), 1.47±0.45 in LBD (p=0.001 vs controls) and1.69±0.28 in MSA (p=0.02 vs controls; p=0.004 vs PD). This ratiowas influenced by PD/LBD diagnosis (ß=–0.638; p<0.001)and to a lesser degree, by ischemic heart disease (ß = –0.244;p=0.028). Optimal cut-off value between PD/LBD and controlswas 1.71 (83% sensitivity and 82% specificity). Within the PDgroup, those with a family history of PD/LB showed higher delayedHMR values (1.65±0.34 vs 1.30±0.24 without history; p<0.001)and proportion with normal scintigraphy (56% vs 5%; p=0.001).Conclusions. Cardiac 123I-MIBG scintigraphy is useful in thediagnosis of Lewy body disorders, although its value in PD isconditioned by having a family history of PD (AU)